Pathogenesis and treatment options for hemophilic synovitis

Abstract

Introduction: Hemophilia is characterized by recurrent bleeding episodes, most commonly in the knees, elbows and ankles. Repeated hemarthroses lead to synovial hypertrophy and a vicious cycle of chronic synovitis arises, leading to destruction of the joint. Areas covered: This article covers the pathogenesis of chronic hemophilic synovitis and its treatment by means of different types of synovectomy. Expert opinion: Both radioynovectomy (RS) and arthroscopic synovectomy considerably improve the frequency of bleeding episodes. RS is the best option for patients with synovitis unresponsive to a three-month trial of hematological prophylaxis. If the bleeding is refractory to three successive episodes of RS at six monthly intervals, arthroscopic synovectomy is indicated. Open synovectomy should be reserved for adults with elbow synovitis requiring radial head removal and synovectomy in the same surgical session. RS is effective and safe, and particularly helpful in patients with inhibitors as they are at greatest risk of bleeding episodes and have the highest risk of complications of surgery.

Haemophilic arthropathy: Contemporary management challenges and a future scenario

“…This phenomenon is called chronic haemophilic synovitis, and it is predisposed to re-bleeding, ultimately leading to a dangerous cycle of haemarthrosis-synovitis-haemarthrosis. 9 If we fail to stop that cycle, the joint cartilage will ultimately be destroyed due to the death of the chondrocytes, advancing within a few years to haemophilic arthropathy 1 (Figure 1). These circumstances substantially impact the quality of life of these individuals, particularly in cases of severe haemophilia (< 1% circulating factor) and in those who present an inhibitor (antibody opposed to the intravenously infused deficient factor).…”